acute chest syndrome of sickle cell anemia

Acute Chest Syndrome of

Sickle Cell Anemia

Ahmed Al Gahtani, BSRC, RRT

Definition

• The acute chest syndrome is a vasoocclusive crisis of

the pulmonary vasculature commonly seen in patients

with sickle cell anemia. The crisis is often initiated by a

lung infection, and the resulting inflammation and loss of

oxygen tension leads to sickling of red cells and further

vasoocclusion.

• ACS is currently defined as a new infiltrate on chest

radiograph in conjunction with 1 other new symptom or

sign: chest pain, cough, wheezing, tachypnea, and/or

fever (> 38.5°C). Platt OS et al, N Engl J Med 2000

Epidemiology

• ACS is a common complication in children with SCD.

• The peak incidence for ACS was in children between two

and four years of age (25.3 per 100 patient-years) with a

higher prevalence during the winter months.

• For patients with SCD, ACS is the second most common

cause of hospitalization (second to vaso-occlusive pain)

with a reported rate of 12.8 hospitalizations per 100

patient /year.

1. Castro, O, Brambilla, DJ, Thorington, B, et al. The Acute Chest Syndrome in Sickle Cell Disease- incidence and

risk factors. Blood 1994.

2. Vichinsky, EP, Neumayr, LD, Earles, AN, Williams, R. Causes and outcomes of the acute chest syndrome in

sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000

Clinical Presentation of ACS

Possible symptoms

• Fever

• Cough

• Chest Pain

• SOB

• Pain in arms or legs

Physical examination

• Rales

• Normal examination

• Fever

• Tachypnea

• Wheezing

• Fever and cough are the most common presenting symptoms in

children, while chest pain, shortness of breath, and chills are common in

adults. Vichinsky EP, Styles LA, Colangelo LH, et al. CSSCD, Blood 1997

Diagnostic Testing in ACS

• Serial chest radiographs

• Consider ventilation and perfusion imaging

• Serial hematologic testing

- Complete blood count

- Reticulocyte count

• Secretory phospholipase A2 measurement if available***

• Arterial blood gas with co-oximetry

• Blood cultures

• Consider bronchoscopy

Aaron W. Bernard, Zahida Yasin, Arvind Venkat, Hospital Physician January 2007

Radiographic features

• New infiltrate most often

involving LL.

• Pleural effusion.

• Pulmonary edema.

• Pulmonary embolism.

• Pulmonary infarction.

CAN J ANESTH; 2003

Therapeutic Modalities for ACS

• Supportive measures: Oxygen for hypoxia, Appropriate

hydration, and Appropriate pain control

• Incentive spirometry

• Antibiotics: Third-generation cephalosporin, macrolides

• Transfusion therapy: Simple transfusion, or Exchange

transfusion

• Experimental therapy: Nitric oxide, or Corticosteroids

Aaron W. Bernard, Zahida Yasin, Arvind Venkat, Hospital Physician January 2007

Case Presentation

• On 22/01/09 @ 2100, a 13 Y/O patient

(318437), known case of SCA brought to ER

(NGH) complaining of lower back pain and left

elbow pain with the following vital signs:

• Body Weight: 36.7 Kg

Temp HR BP RR Sat

37.2 89 110/53 20 96% on RA

Patient History

• HPI:

Pt was well until one day ago when he started to have

lower back and left elbow pain, pain started suddenly.

With NO fever, respiratory symptoms, abdomen pain, or

any other symptoms.

• PMH:

The Pt is known case of Sickle Cell Anemia diagnosed at

the age of 5 years. With two previous hospitalization first

was on July, 2006 due to aplastic crisis and headache

(vaso-occlusive crisis) the second was on Jan, 2007 with

admitting diagnosis of SCA with Acute Chest Syndrome

versus pneumonia.

• Allergies: No Known Drug Allergy.

Patient History

• Past Surgical History: None

• Family Medical History: Mother and one of his sisters

is SC trait. Parents are related of the second degree.

• Psychosocial History: Immunization completed, Child,

Student.

• Developmental History: Appropriate to age.

• Nutritional History: The patient is on family diet with

good appetite.

Pain Assessment

• Current continuous pain for one day.

• Location: Lower back + Left elbow.

• Pain Score: 8/10

Physical Exam

• General Appearance: Look well, not under distress,

in pain, well hydrated.

• Heent: Clear and normal.

• Chest: Clear BS with equal E/A bilaterally.

• CVS: Normal Sinus Rhythm, Regular with normal heart

sounds S1+S2.

• Abdomen: Soft, Bowl Sound Present.

• Neurological: Grossly Intact.

• Musculoskeletal: Pain.

Labs

TYPE 22/01/2009 23/01/2009 NORMAL

WBC 13.9 17.5 (4000 – 11500)/mm3

RBC ----- 2.92 (4.35 -5.55)X10^6/mm3

Hgb 84 81 (125 -155) G/L

Hct ----- .229 (.360 - .460)

Plt 771 693 (150 – 400)X10^3/mm3

Lymph % 31 27 26% - 46%

Mono % 11 9 3% - 9%

Neut % 57 58 34% - 64%

Bands % ----- 1 0% - 6%

Labs

TYPE 23/01/2009 NORMAL

BUN 2.4 (3.0 – 7.5) MMOL/L

Sodium 135 (136 – 145) MMOL/L

Potassium 4.8 (3.5 – 5.1) MMOL/L

Chloride 101 (95 – 110) MMOL/L

CO2 20 (20 – 28) MMOL/L

Glu R 5.8 (2.9 – 7.8) MMOL/L

Crea 44 (44 – 88) MMOL/L

Assessment & Plan

• Assessment: 13 y/o boy with SCA came with

crisis.

• Plan: Admission, Hydration, Pain Control, and

start Antibiotic therapy if Pt develop fever.

Day 2, 24/Jan/2009

• Patient still in the ER.

• General: Patient looks under moderate to severe

respiratory distress.

• V/S:

• HEENT: Congested throat.

• CVS: Sinus Tachycardia, Regular with normal heart

sounds S1+S2.

• Abdomen: Soft, Bowl Sounds present.

• Labs: No significant changes, WBC 13.9, Hgb

electrophoresis Positive.

Temp HR BP RR Sat

39.0 124 126/73 35 91% on 10LPM via NRFM

Day 2, Cont.

• Respiratory: Tachypnic, with increased oxygen

requirement, and increased WOB.

- Chest: Subcostal and Suprasternal retractions.

- BS: Bilateral Insp. & Exp Crackles with decreased A/E.

- CXR: No previous films for comparison, airspace

consolidation involving apical and posterior segments of

LLL associated with mild left PE and minimal right PE.

Airspace infiltrate in the rests of LLL and at the right

base.

- ET Culture: Negative, no organisms seen.

Day 2, Cont.

• Assessment: ACS of SCA vs. Pneumonia

• Plan:

- Pt to be admitted to PICU as soon as possible.

- Broad Spectrum Antibiotics.

- Simple transfusion, if no good outcome then Exchange

transfusion.

- IV hydration with analgesics.

• Medications:

Morphine Sulfate, Ibuprofen, Ranitidine Hydrochloride,

and Ceftriaxone Sodium.

• @ 2000 of the same day (24/1) patient

was brought to PICU on 10 LPM via

NRFM. BS bilateral coarse crackles with

decreased A/E. CXR showed lower lobes

consolidation and infiltrates. With the

following V/S:

Temp HR BP RR Sat

38.5 94 115/65 32 90%

Day 3, 25/Jan/2009

• CNS: GCS 7/15, on Midazolam and Morphine infusion.

• CVS: Sinus Tachycardia, Regular with normal heart sounds

S1+S2. Central line inserted in right femoral.

• V/S:

• Labs:

Temp HR BP RR Sat

38.5 121 139/66 30 90% on 10 LPM via NRFM

Type 0533 1547 2200 Type 0533 1547 2200

WBC 28.9 23.0 32.6 Plt 214 220 262

RBC 3.81 3.47 3.16 Lymph% 12% 8% 14%

Hgb 109 98 89 Bands% ---- 14% 19%

Hct .306 .278 .254 Neut% 73% 61% 45%

Day 3, Cont.

• Respiratory: Increased WOB, Tachypnic, BS were

bilateral Insp. & Exp. Crackles with decreased A/E. Pt

continue to desat below 87% on NRFM. So he was

placed on BiPAP 15/8, FiO2 0.8 via face mask @ 0145.

- ABG on BiPAP:

pH PCO2 PO2 HCO3 BE Sat

7.32 51.6 71.2 26.3 - 0.1 94%

Day 3, Cont.

• Comparison was made to study dated

24/01/09. Worsening.

• On the right side, atelectasis of RML &

RLL with moderate PE.

• On the left side, mild PE.

• Patchy opacities are seen in both upper

lobes.

• The cardiac outline is obscured by

dense opacification of both

hemithoraces.

• No air leak is seen.

Day 3, Cont.

• Respiratory: Cont.

- @ 0630 Pt continue to desat on BiPAP 20/12 and FiO2

1.0 so Pt was intubated with cuffed ETT size 6.5 with no

procedural complications. Then he was placed on PCV

mode via Servo i on the following settings:

- ABG @ 1030 showed:

- So PC was increased to 24 and PEEP to 17.

Set Parameters Measured Parameters

PC PEEP RR FiO2 Ti Ppeak MAP VTe SpO2

22 12 22 1.0 1.1 35 23 240 91%


7.09 107 34.5 26.6 - 4.6 91%/76%

Day 3, Cont.


- Right Chest Tube was inserted.

- Multiple episodes of bagging & suctioning to maintain

acceptable saturation.

- Pt developed right pneumothorax . So the right chest tube

was repositioned and a left chest tube was also inserted.

- @ 1145, HFOV trail was failed due to hemodynamic

instability.

- @ 1240, Pt was placed on CMV (PCV) via Servo i:



35 19 22 1.0 1.1 54 32 384 83%

@ 0853 @ 2103

Day 3, Cont.


- ABG @ 1519:

so RR was increased to 25 and PC decreased to 24.

- iNO was started 25ppm by 0.2 LPM flow.

- ABG @ 2210:

so RR decreased to 22, PC decreased to 22, PEEP to 18,

and FiO2 t0 0.9

- Plan is to continue monitoring Pt closely, keep SpO2 > or =

92%.


7.21 56.5 34.4 22.1 - 5.8 84%/59%


7.39 37.4 67.4 22.6 - 2.8 94%/93%

Day 3, Cont.

• Medications:

- Midazolam.

- Fentanyl.

- Rocuronium.

- Morphine Sulfate

- Dopamine.

- Dobutamine.

- Ibuprofen.

- Ceftriaxone Sodium.

- Ranitidine Hydrochloride.

- Azithromycin.

- Vancomycin Hydrochloride.

- Folic Acid.

- D5 in NaCl 0.45%, 500 ml

with 10 mEq Potassium.

- Ventolin.

Day 4, 26/Jan/2009

• CNS: Pt on Morphine 30mg/Kg/h, Fehtanyl PRN, Midazolam

was stopped. Pt pupils react 2-3 mm and moving all limbs,

GCS 10/15.

• CVS: Normal Sinus Rhythm, Regular with normal heart sounds

S1+S2. Plan is to started weaning process from Inotropes

maintaining HR 85-110, BP 110-130/60-70s (80-95).

• V/S:

Temp HR BP RR SpO2

38.5 98 130/64 22 93%

Day 4, Cont.

• Respiratory:

- BS: bilateral fine crackles with diminished breath sounds

over both bases.

- CXR: airspace infiltrates in both upper lobes, PE, and

possible left lower lobe collapse.

- Bag/Suction: moderate, loose, and white ( done once)

- Oxygenation Management: FiO2 and PEEP were both

decreased gradually FiO2 0f 0.6 and PEEP of 10,

maintaining SpO2 above 92%.

- Third Chest Tube was inserted on the left.

Day 4, Cont.

• Respiratory: cont.

Ventilation Management:

- @ 0150 vent settings were as follow:

- iNO: no change.

- ABG @ 1624



22 18 22 0.8 1.1 40 26 234 94%


7.38 35 88.9 20.5 - 4.5 96%/97.5%

Day 4, Cont.



- @ 1630 vent settings were changed to the following:

- They continue to wean the PEEP to 14 then 12 based on

SpO2.

- ABG @ 2209:



22 16 20 0.6 1.1 38 24 278 95%


7.43 36.9 101 24.0 0.3 96%/98%

Day 4, Cont.



- @ 2320 vent settings were changed to the following:

The plan for RCP is maintain ventilation to keep SpO2 > or =

88 - 92%, Normal pH, Q8h ABG, Q4h Ventolin.



22 10 20 0.6 1.1 31 19 261 96%

Day 4, Cont.

• Medications:

- Epinephrine ( Vasopressin).

- Meropenem.

- Dexamethazone. 10mg IV q12h for 2 days

- Acetaminophen.

Day 7, 29/Jan/2009

• CNS: GCS 11/15, Pt on Morphine 30mg/Kg/h, Fehtanyl PRN.

• CVS: Pt is stable.

• V/S:

• Respiratory: bilateral fine crackles with fair A/E. CXR showed

no significant change. iNO was weaned to 8ppm by none but

was increased to 15ppm at 1600. FiO2 was 0.9 in the morning

then decreased to 0.65 at 1440 but at 1550 was increased to

0.9. Pt was bagged & suctioned twice giving small amount of

thick white secretion. Pt still has 3 chest tubes 2 on the left

and 1 on the right.

Temp HR BP RR SpO2

36.7 100 130/67 31 96%

Day 7, Cont.


Ventilation Management: @ 0725

@ 1207

@ 1550

@ 1733


SPC PEEP PS FiO2 RR Ti Ppeak MAP VTe SpO2

26 14 12 0.9 20 1.1 27 28 180 96%


7.44 45.1 71.3 30.2 6.1 98%/95%


VCVT PEEP FiO2 RR Ti Ppeak MAP VTe SpO2

270 10 1.0 30 0.75 40 19 231 93%


7.43 45.1 52.8 29.8 5.3 88%/85%

Day 10, 1/Feb/2009

• CNS: Pt is sedated, GCS 7/15.

• CVS: Pt is stable and off inotrops .

• V/S:

• Labs: WBC 30.6, Hgb 105, Plt 921, Na+ 144, K+ 4.7,

CO2 32, Cl- 103, Crea 42.

• Respiratory: BS were bilateral fine crackles with expiratory

wheezes. The chest tube #4 was reinserted at 1012, iNO was

weaned off, and Pt was bagged and suctioned for 6 times

during this day giving moderate amount of yellow thick

secretions. Plan is to keep SpO2 > or = 88 – 92% and blood

gas q8h.

Temp HR BP RR SpO2

37.4 94 117/72 33 93%

Day 10, Cont.



@ 1017

ABG @ 1129

@1200


PRVCVT PEEP FiO2 RR Ti Ppeak MAP VTe SpO2

270 13 1.0 30 0.75 42 23 253 91%


7.42 50.6 71.9 32.3 7.8 90%/97%


PRVCVT PEEP FiO2 RR Ti Ppeak MAP VTe SpO2

270 10 0.9 30 0.75 38 22 226 90%

Day 10, Cont.

Day 10, Cont.

• Medications:

- Folic Acid, 1mg tab, q am

- Potassium Chloride, 5 mEq via tube q12h

- Ranitidine, 30 mg IV/30 min, q8h

- Ibuprofen, 300 mg, q6h

- Acetaminophen, 500 mg IVP, q6h

- Furosemide, 10 mg IVP, q8h

- Ventolin, 6 puffs/ q4h

- Midazolam, 2 mcg/kg/hr

- Morphine, 55 mcg/kg/hr

Day 25, 16/Feb/2009

• General: Pt was placed on prone position during this day.

• CNS: Pt is sedated, GCS 3/15.

• CVS: Sinus Tachycardia, Regular with normal heart sounds

S1+S2. Inotrops were restarted.

• V/S:

• Labs: WBC 49, Hgb 91, Plt 1625, Na+ 141, K+ 4.4,

CO2 30, Cl- 102, Crea 33.

• Respiratory: Over the night Pt developed left pneumothorax

and it was difficult oxygenate and ventilate with PCO2

accumulation so Pt was shifted to HFOV. The fifth chest tube

was inserted on the left side. Endotracheal culture result

showed positive yeast growth.

Temp HR BP RR SpO2

38.1 148 100/90 35 89%

Day 25, Cont.



ABG

ABG

0030 Set Parameters Measured Parameters

Mode PC PEEP RR FiO2 Ti P peak MAP VTe SpO2

PC 39 13 30 1.0 0.75 49 30 200 85%

Time pH PCO2 PO2 HCO3 BE Sat

0058 7.17 107 61.1 38.5 10.0 81%/85%

0100 Set Parameters Measured Parameters

Mode PC PEEP RR FiO2 Ti P peak MAP VTe SpO2

PC 35 12 35 1.0 0.55 85%


0347 7.15 124 65.5 42.7 13.9 85%/89%

Time Set Parameters

0415Mode Hz ∆P MAP Ti% Flow FiO2

HFOV 8 66 32 0.33 20 1.0

Day 25, Cont.

ABG

ABG

ABG

ABG


0514 7.03 167 56.8 43.3 13.0 74%/81%

Time Set Parameters


HFOV 6 68 35 0.33 20 1.0


0514 7.08 140.5 52.3 40.3 10.2 74%/81%

Time Set Parameters


HFOV 5 70 34 0.33 20 1.0


0737 7.23 95.4 50.7 38.9 11.4 88%/80%


2215 7.27 88.9 67.2 40.0 13.1 90%/92%

Day 25, Cont.

Day 25, Cont.

• Medication:

- Medazolam, 5 mcg/kg/hr.

- Fentanyl, 8 mcg/kg/hr.

- Precedex, 0.8 mcg/kg/hr.

- Cisatracurium, 3 mg/kg/hr.

- Epinephrin, 0.1 mcg/kg/min.

- Dobamine, 20 mcg/kg/min.

- Dobutamine, 16 mcg/kg/min.

- Lasix, infusion.

Day 36, 2/March/2009

• General: Pt is on supine postion since the 20th of last month.

• CNS: Pt is sedated, GCS 6/15, on same sedatives as on day

25.

• CVS: Pt is tachycardic but stable, and off inotrops support.

• V/S:

• Labs: WBC 23.7, Hgb 100, Plt 651, Na+ 144, K+ 3.9,

CO2 37, Cl- 97, Crea 30

• Respiratory: Good wiggle, 6 chest tubes (3 on right, and 3 on

the left). ICU team inform patient’s family about his lungs

status and its poor prognosis, the team are planning to switch

the patient back to CMV. Endotracheal culture done on the

28/2/2009 and the result was negative

Temp HR BP RR SpO2

36.6 137 108/41 86%

Day 36, Cont.



VBG

No changes was done on the vent settings, only FiO2 was

titrated to keep SpO2 within the accepted range.

Time Set Parameters


HFOV 5 60 25 0.33 20 1.0


1007 7.31 83.6 48.7 41.7 15.5 100%/81%

Day 36, Cont.

Day 36, Cont.

• Medication:

- Voriconazole.

- Metoclopramide.

THANK YOU

ANYQUESTIONS

acute chest syndrome of sickle cell anemia

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